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BACKGROUNDMyocardial work (MW) is an index of LV function based on pressure–strain loops and brachial cuff pressure measurement. MW has been proposed as more sensitive than conventional functional parameters, as it accounts for afterload and myocardial deformation. However, many studies have been limited to assessment of global MW indices, neglecting regional differences in cardiac associated with hypertension and consequent cardiac remodeling. We aimed to quantify regional MW in pediatric hypertension and compare the findings in renal or renovascular hypertension (RHTN) with essential hypertension (EHTN).METHODSWe retrospectively assessed conventional markers of LV function, and both global and regional MW indices in 78 patients (49 males, 15.4 ± 2.94 years) with EHTN and RHTN.RESULTSPeak systolic strain (PSS) in the basal septal segment was significantly impaired in patients with RHTN compared to EHTN (−13.00% [−15.50%; −13.00%] vs. −15.00% [−17.50%; −13.50%], P = 0.034). Similarly, basal septal MW indices were significantly elevated in patients with EHTN compared to RHTN, including MW efficiency (MWE) (95.0% [93.0%; 98.0%] vs. 94.0% [89.0%; 95.0%], P = 0.004) and constructive work (CW) (1700 mm Hg% (409 mm Hg%) vs. 1520 mm Hg% (336 mm Hg%), P = 0.037). Wasted work (WW) was significantly elevated in the RHTN group (79.0 mm Hg% [28.5 mm Hg%; 104 mm Hg%] vs. 105 mm Hg% [62.0 mm Hg%; 164 mm Hg%], P = 0.010).CONCLUSIONSignificant differences in basal septal PSS and MW indices were observed between EHTN and RHTN. These findings highlight the usefulness of regional MW indices in assessing disease and may help differentiate between etiologies of pediatric hypertension.

Left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) have conventionally been used for surveillance of cardiac function after cancer therapy, but indices of myocardial work (MW) are potentially superior for this purpose because they take into account both myocardial deformation and loading conditions.We aimed to investigate the usefulness of MW in the follow-up of children and young adults following anthracycline chemotherapy.Conventional markers of LV function (LV fractional shortening [LVFS], LVEF, GLS) and MW indices (global work index [GWI], global constructive work [GCW], global wasted work [GWW], and global work efficiency [GWE]) were obtained from 2342 echocardiographic examinations in 598 patients (354 male, 12.2 [4.7-17.3] years at initiation of chemotherapy).GWI, GCW, GLS, LVFS, and LVEF all deteriorated significantly during and after anthracycline chemotherapy, while GWW decreased and GWE was preserved. On multivariable analysis, MW indices were correlated with conventional markers of LV function and with clinical information relating to underlying malignancy and chemotherapy. Cox regression analysis revealed that similar levels of deterioration in GWW, GWI, and GCW preceded those in GLS, LFS, and LVEF.Non-invasive MW indices correlate well with conventional markers of LV function. Indices of MW appear to provide an earlier and more sensitive marker of progression towards chemotherapy-related cardiac dysfunction. Future studies are warranted to validate whether the incorporation of non-invasive MW into the routine clinical surveillance in patients after chemotherapy would improve outcomes.

Background Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)–left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). Methods Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure–volume catheterization. Results In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL−1; 95% CI, -0.02 to 0.09 mL−1). Conclusions The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function.

Background: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. Methods: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. Results: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5–62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). Conclusions: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

AimsHypoplastic left heart syndrome (HLHS) survival relies on surgical reconstruction of the right ventricle (RV) to provide systemic circulation. This substantially increases the RV load, wall stress, maladaptive remodelling, and dysfunction, which in turn increases the risk of death or transplantation.Methods and resultsWe conducted a phase 1 open-label multicentre trial to assess the safety and feasibility of Lomecel-B as an adjunct to second-stage HLHS surgical palliation. Lomecel-B, an investigational cell therapy consisting of allogeneic medicinal signalling cells (MSCs), was delivered via intramyocardial injections. The primary endpoint was safety, and measures of RV function for potential efficacy were obtained. Ten patients were treated. None experienced major adverse cardiac events. All were alive and transplant-free at 1-year post-treatment, and experienced growth comparable to healthy historical data. Cardiac magnetic resonance imaging (CMR) suggested improved tricuspid regurgitant fraction (TR RF) via qualitative rater assessment, and via significant quantitative improvements from baseline at 6 and 12 months post-treatment (P < 0.05). Global longitudinal strain (GLS) and RV ejection fraction (EF) showed no declines. To understand potential mechanisms of action, circulating exosomes from intramyocardially transplanted MSCs were examined. Computational modelling identified 54 MSC-specific exosome ribonucleic acids (RNAs) corresponding to changes in TR RF, including miR-215-3p, miR-374b-3p, and RNAs related to cell metabolism and MAPK signalling.ConclusionIntramyocardially delivered Lomecel-B appears safe in HLHS patients and may favourably affect RV performance. Circulating exosomes of transplanted MSC-specific provide novel insight into bioactivity. Conduct of a controlled phase trial is warranted and is underway.Trial registration number NCT03525418.

Prior to the 1990s, d-TGA was palliated with the atrial switch procedure resulting in a systemic right ventricle associated with significant long-term morbidity and mortality. Determining the optimal timing of heart transplantation (HT) in these patients has been difficult. While cardiopulmonary exercise testing (CPET) is commonly used to try and risk stratify these patients, traditional exercise parameters have lacked the sensitivity and specificity to assess long-term risk. We sought to assess changes in exercise parameters over time in order to determine if any CPET parameter or combination of parameters could reliably identify risk for adverse outcome in this patient group. A retrospective review of serial CPET for 40 patients over 17 years was completed. Patients with adverse event within 6 months prior to CPET were noted. CPET parameters were compared and linear mixed model regression with repeated measures was performed on serial tests for longitudinal assessment. The linear mixed model regression identified OUES indexed to BSA to be the most sensitive parameter in identifying patients at risk of adverse event and became a stronger predictor of adverse event when combined with peak heart rate. CPET is useful in identifying patients with atrial switch at increased risk of adverse outcome. Indexed OUES and peak heart rate are better prognostic indicators than VO2 and VE/VCO2.

Background The aims of this study were to investigate the dynamic changes in the vena contracta (VC) and proximal isovelocity surface area (PISA) through systole in patients with hypoplastic left heart syndrome and tricuspid regurgitation and to identify the stage of systole (early, mid, or late) in which VC and PISA radius are optimal. Methods Twenty-eight patients with hypoplastic left heart syndrome were prospectively studied using continuous two-dimensional (2D) and three-dimensional (3D) echocardiography. Two-dimensional VC width, 3D VC area, and PISA radii (2D and 3D) were measured frame by frame throughout systole. The maximal 2D VC width, 3D VC area, and PISA radii in the first, middle, and last thirds of systole were compared, and correlations were explored with 3D tricuspid annular areas, right atrial volumes, and right ventricular volumes. Results In all, 35 data sets that met inclusion criteria were analyzed. On frame-by-frame analysis, maximal 2D VC width and 3D VC area were found in the first third of systole in 17% and 20% of studies, in the second third in 34% and 31%, and in the final third in 49% and 49%. Similarly, the maximal 2D and 3D PISA radii were found in the first third of systole in 26% and 17% of studies, in the second third in 28% and 34%, and in the final third in 46% and 49%. Conclusions In hypoplastic left heart syndrome, detailed temporal analysis of tricuspid regurgitation–associated VC and PISA by 2D and 3D echocardiography reveals no reliable pattern predicting when in systole these parameters peak. Frame-by-frame measurement is necessary for identification of maximal VC and PISA radius on 2D and 3D color Doppler echocardiography because the severity of tricuspid regurgitation could be underestimated because of temporal variability in VC and PISA.

Heart murmur, a thoracic auscultatory finding of cardiovascular origin, is extremely common in childhood and can appear at any age from premature newborn to late adolescence. The objective of this review is to provide a modern examination and update of cardiac murmur auscultation in this new era of artificial intelligence (AI) and telemedicine. First, we provide a comprehensive review of the causes and differential diagnosis, clinical features, evaluation, and long-term management of paediatric heart murmurs. Next, we provide a brief history of computer-assisted auscultation and murmur analysis, along with insight into the engineering design of the digital stethoscope. We conclude with a discussion of the paradigm shifting impact of deep learning on murmur analysis, AI-assisted auscultation, and the implications of these technologies on telemedicine in paediatric cardiology. It is our hope that this article provides an updated perspective on the impact of AI on cardiac auscultation for the modern paediatric cardiologist.

BackgroundHypoplastic left heart syndrome (HLHS) survival relies on surgical reconstruction for the right ventricle (RV) to provide systemic circulation. This leads to substantially increased loads on the RV, wall stress, maladaptive remodeling and dysfunction, which in turn can increase risk of death or transplantation.ObjectivesWe conducted a phase I multicenter trial to assess safety and feasibility of intra-operative MSC injection in HLHS patients to boost RV performance in the systemic position.MethodsAllogeneic MSCs were directly administered by intramyocardial injections during the second stage palliative operation. The primary endpoint was safety.ResultsTen patients received intramyocardial injections of allogeneic MSCs (Lomecel-B). No patients experienced major adverse cardiac events (MACE). All subjects were alive and transplant-free at 1 year following, and experienced growth comparable to healthy control historical data. Cardiac magnetic resonance imaging (CMR) revealed improving tricuspid regurgitant fraction (Baseline: 0.45±0.19; 6 mo.: 0.32±0.06; 12 mo.: 0.06±0.09), while global longitudinal strain (Baseline: -24.39±6.99; 6 mo.: -20.55±3.05, p > 0.05 vs baseline; 12 mo.: - 23.88±4.6, p>0.05 vs baseline) and RV ejection fraction (EF; baseline: 62.62±5.99; 6 mo.: 53.69±9.56; 12 mo.: 52.31±5.63: p=NS for change over time) were unchanged. Computational modeling identified 167 derived RNAs specific to circulating exosomes originating from transplanted MSCs corresponding to RVEF changes and identifying potential mechanistic underpinnings.ConclusionsIntramyocardial MSCs appear safe in HLHS patients, and may favorably affect RV performance. Circulating exosomes of transplanted MSC-specific provide novel insight into bioactivity. Conduct of a controlled phase trial is warranted and is underway.Condensed AbstractThe ELPIS phase I trial was designed to assess safety and feasibility of intramyocardial injection of allogeneic MSCs into the RV during second stage palliation of HLHS. There were no incidences of major adverse cardiac events (MACE) or other safety concerns, and there was a 100% transplant-free survival at 1-year follow-up, supporting the safety and feasibility of this approach. The ELPIS results are important for advancing MSC therapy for all ages and congenital heart conditions, and warrant further investigation in a controlled Phase II trial powered for efficacy.

Objective The objective of this study was to assess papillary muscle (PM) and mitral valve (MV) structure and function in children and young adults with mild and moderate hypertrophic cardiomyopathy (HCM) using real-time three-dimensional echocardiography (3DE) and to correlate them with HCM related adverse outcomes. Methods Transthoracic research 3DE was performed in HCM patients and controls matched for age and gender. Anterolateral and posteromedial PM mass, apical displacement of anterolateral PM, and left ventricular (LV) mass were measured and indexed to body surface area. The MV annulus and leaflet structure and function were analyzed. Individual PMs were manually planimetered by tracing the endocardial borders on each mid systole frame, taking care to distinguish PMs as distinct from the LV wall. Apical PM displacement was expressed as ratio of the distance between the apex and the base of the anterolateral PM to the entire length of the LV lateral wall (APL index). All 3DE measurements were correlated to adverse outcomes. Results Forty subjects were studied, including 20 HCM patients (age 18.1 ± 9.6 years, 16 male and 4 female), and 20 controls (18.2 ± 9.6 years, 16 male and 4 female). The indexed LV mass in HCM was 74.8 ± 25.8 g/m2 compared to 50.8 ± 12.4 g/m2 in controls (p = 0.001). The anterolateral, posteromedial and combined PM mass were 3.1 ± 2.2 g/m2, 1.7 ± 1.2 g/m2 and 4.9 ± 2.7 g/m2 in HCM, in contrast to respective measurements of 1.1 ± 0.6 g/m2, 1.2 ± 0.6 g/m2 and 2.3 ± 0.8 g/m2 in controls (p Conclusion It is feasible to evaluate PM muscles and MV annulus geometry in children and young adults with HCM using 3DE. The morphologic and functional changes of anterolateral PM may occur in the absence of MV annulus changes. Prospective validation will be required to determine if LV lateral wall length and LV mass may be used as predictors of adverse events.

BACKGROUND The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplantation has been the only option. This study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients. METHODS We conducted a retrospective study of Fontan patients in the Advanced Cardiac Therapies Improving Outcomes Network. We evaluated patient characteristics, and the clinical and physiologic outcomes after VAD implantation. RESULTS We identified 45 Fontan patients implanted with VAD. The average age of patients was 10 years (interquartile range: 4.5–18) and 30% were female. The majority had a morphologic right ventricle (69%), moderate or greater ventricular dysfunction (83%), and moderate or greater atrioventricular valve regurgitation (65%). The majority of implants were as a bridge to transplantation (76%), and the majority of patients were Interagency Registry for Mechanically Assisted Circulatory Support Profile 2 (56%). The most commonly employed device was the Medtronic HeartWare HVAD (56%). A total of 13 patients were discharged on device support, and 67% of patients experienced adverse events, the most common of which were neurologic (25%). At 1 year after device implantation, the rate of transplantation was 69.5%, 9.2% of patients continued to be VAD supported, and 21.3% of patients had died. Hemodynamically, VAD was effective in decreasing both Fontan and ventricular end-diastolic pressures in some individuals. CONCLUSIONS VAD is effective in supporting patients with end-stage Fontan failure awaiting heart transplantation. Future research should focus on identifying clinical and physiologic characteristics predictive of a favorable response to VAD support.

BACKGROUND We implemented sophisticated color M-mode analysis to assess age-dependent progression of left ventricular (LV) diastolic function. METHODS Normal infants were prospectively enrolled for serial echocardiograms at 1 week, 1 month, 6 months, 1 year, and 2 years. From color M-mode scans, propagation velocity (VP), strength of filling (VS), and intraventricular pressure difference (IVPD) in 3 segments along apex-to-mitral valve scan line were measured. RESULTS Age-wise comparisons of diastolic filling from 121 echocardiograms in 31 infants showed VP (cm/s), VS (cm2/s), and E-wave IVPD (mmHg) at 1 week to be 66.2 ± 11.9, 75.3 ± 19.9, and 1.5 ± 0.4, respectively, while VP, VS, and E-wave IVPD at 1 month were 80.3 ± 14.4, 101.2 ± 28.3, and 2.42 ± 1.1, respectively. There were significant differences in VP and segmental IVPD between first week and first month (p

Myocardial work (MW) is a novel parameter that incorporates non-invasive estimates of left ventricular (LV) systolic pressure into the interpretation of strain, thus overcoming the limitations of load dependency traditionally encountered with LV fractional shortening (LVFS), ejection fraction (LVEF), and global longitudinal strain (GLS). However, data on MW in the pediatric population with hypertension are lacking. Conventional markers of LV function and MW indices were obtained from 88 echocardiographic examinations in 76 children with hypertension (47 males, 15.5 ± 2.96 years). When compared with a previously published cohort of 52 healthy controls, global work index (GWI) and global constructive work (GCW) were both significantly elevated while LVEF and GLS were not impaired but rather mildly increased. On multivariable analysis, GWI was correlated with systolic blood pressure (slope = + 16, p 0.001) and GLS (slope = -100, p 0.001), while GCW was correlated with systolic blood pressure (slope = + 18, p 0.001), GLS (slope = -101, p 0.001), male sex (slope = -75, p = 0.016), and LV mass (slope = -0.93, p 0.001). Global wasted work (GWW) was correlated with age at echo visit (slope = -4.5, p = 0.005) and GLS (slope = + 5.5, p 0.001). The opposite occurred for global work efficiency (GWE; slope = + 0.20, p = 0.011, and slope = -0.48, p 0.001, respectively). Principal component analysis and k-means clustering revealed 4 subphenotypes which differed in terms of etiology, afterload, and compensation stage of the disease. Conclusion: Non-invasive MW shows a good correlation with conventional markers of LV function and may help refine the assessment of hypertensive heart disease in children. What is Known: • Myocardial work (MW) is a novel parameter that incorporates non-invasive estimates of left ventricular (LV) systolic pressure into the interpretation of strain, thereby accounting for both deformation and afterload. • Although the usefulness of MW in the assessment of myocardial function beyond conventional markers has been demonstrated in various clinical populations, data in the pediatric population with hypertension are currently lacking. What is New: • Compared to normal values in healthy children, global work index and global constructive work were increased in those with hypertension, while LV ejection fraction and global longitudinal strain were not impaired. • Machine learning identified 4 subphenotypes which differed in terms of etiology, afterload, and compensation stage of the disease.

Background Pulmonary arterial end‐diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive physiology, but is not fully understood. This systematic review and meta‐analysis sought to clarify its physiological and clinical correlates, and to define a framework for understanding EDFF and RV restrictive physiology. Methods and Results PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for observational studies published before March 2021. Random‐effects meta‐analysis was performed to identify factors associated with EDFF. Forty‐two individual studies published between 1995 and 2021, including a total of 2651 participants (1132 with EDFF; 1519 with no EDFF), met eligibility criteria. The pooled estimated prevalence of EDFF among patients with repaired tetralogy of Fallot was 46.5% (95% CI, 41.6%–51.3%). Among patients with EDFF, the use of a transannular patch was significantly more common, and their stay in the intensive care unit was longer. EDFF was associated with greater RV indexed volumes and mass, as well as smaller E‐wave velocity at the tricuspid valve. Finally, pulmonary regurgitation fraction was greater in patients with EDFF, and moderate to severe pulmonary regurgitation was more common in this population. Conclusions EDFF is associated with dilated, hypertrophied RVs and longstanding pulmonary regurgitation. Although several studies have defined RV restrictive physiology as the presence of EDFF, our study found no clear indicators of poor RV compliance in patients with EDFF, suggesting that EDFF may have multiple causes and might not be the precise equivalent of RV restrictive physiology.

PURPOSE OF REVIEW Artificial intelligence (AI) has changed virtually every aspect of modern life, and medicine is no exception. Pediatric cardiology is both a perceptual and a cognitive subspecialty that involves complex decision-making, so AI is a particularly attractive tool for this medical discipline. This review summarizes the foundational work and incremental progress made as AI applications have emerged in pediatric cardiology since 2020. RECENT FINDINGS AI-based algorithms can be useful for pediatric cardiology in many areas, including: (1) clinical examination and diagnosis, (2) image processing, (3) planning and management of cardiac interventions, (4) prognosis and risk stratification, (5) omics and precision medicine, and (6) fetal cardiology. Most AI initiatives showcased in medical journals seem to work well in silico, but progress toward implementation in actual clinical practice has been more limited. Several barriers to implementation are identified, some encountered throughout medicine generally, and others specific to pediatric cardiology. SUMMARY Despite barriers to acceptance in clinical practice, AI is already establishing a durable role in pediatric cardiology. Its potential remains great, but to fully realize its benefits, substantial investment to develop and refine AI for pediatric cardiology applications will be necessary to overcome the challenges of implementation.

The aim of this study was to investigate the impact of the new United Network for Organ Sharing (UNOS) listing criteria on mechanical circulatory support (MCS) utilization and outcomes in adult congenital heart disease (ACHD) patients.We identified all ACHD and non-ACHD heart transplant candidates in the Scientific Registry of Transplant Recipients database listed during the 590 days prior to (historical cohort) or following (recent cohort) the UNOS allocation revision on October 18, 2018. Patients were grouped based on whether they received central temporary MCS (tMCS), peripheral tMCS, durable MCS, or no MCS.A total of 535 ACHD (242 historical, 293 recent) and 12,188 non-ACHD (6,258 historical, 5,930 recent) patients were included in our study. For ACHD patients, we found no differences in the historical versus recent cohort in utilization of central tMCS (3.31% vs 3.07%, p = .88) or durable MCS (3.31% vs 3.41%, p = .95), whereas the rate of peripheral tMCS increased (2.07% historical vs 6.83% recent, p = .009). Across both cohorts, ACHD patients supported with peripheral tMCS had shorter time-to-transplant than non-supported patients (25.7 vs 121.7 days, p = .002). ACHD patients supported with central tMCS had greater rates of post-transplant mortality relative to other ACHD patients (40.0% vs 12.6%, p = .006), while those supported with durable or peripheral temporary MCS had no differences in waitlist or post-transplant mortality compared to non-supported ACHD patients.The 2018 UNOS allocation changes increased utilization of peripheral temporary MCS in ACHD patients, decreasing waitlist time without impact on post-transplant outcomes.

Background Patients with adult congenital heart disease (ACHD) experience long waitlist times for heart transplantation (HTx) while a large proportion of donor hearts are refused. The goal of this study was to inform optimal donor selection for patients with ACHD listed for HTx by examining the impact of donor characteristics on post‐HTx outcomes. Methods and Results Using the Scientific Registry of Transplant Recipients, we conducted a retrospective analysis of patients aged ≥18 years listed for HTx in the United States between 2000 and 2016. We compared waitlist times between patients with ACHD and patients with noncongenital heart disease and constructed multivariate hazard models to identify donor characteristics associated with increased waitlist time. We then compared post‐HTx survival between patients with ACHD and patients with noncongenital heart disease and constructed multivariate hazard models to identify donor characteristics associated with mortality. There were very few differences in donor characteristics between HTx recipients with ACHD and those with noncongenital heart disease. Status 1A–listed patients with ACHD experienced longer waitlist times compared with patients with noncongenital heart disease. Increased wait times were associated with some donor characteristics. Post‐HTx outcomes varied over time, with patients with ACHD having inferior early mortality (0 to 30 days), similar intermediate mortality (31 days to 4 years), and superior late mortality (>4 years). We identified no donor characteristics associated with mortality to justify the observed differences in donor selection or waitlist time. Conclusions HTx candidates with ACHD wait longer for transplant but do not require unique donor selection criteria. HTx teams should consider liberalizing donor criteria and focusing only on evidence‐based selection to improve waitlist outcomes and reduce the recipient–donor disparity.

Objective:This prospective observational study evaluates the utility of expert focused cardiac ultrasound (eFCU) with spectral Doppler to inform decision making in established patients with specific, selected congenital cardiac defects in outreach clinics. Secondary objectives include determining if the addition of eFCU expands capacity in paediatric cardiology outreach clinics and if it improves the patient experience.Methods:Patients aged 2 months to 19 years old with a diagnosis of ventricular septal defect, atrial septal defect, atrioventricular septal defect, patent ductus arteriosus, aortic valve stenosis, or pulmonary valve stenosis with the need for follow-up echocardiography in an outreach clinic from August 2017 to June 2018 were studied. A novel assessment tool was used to determine the success of eFCU.Results:Forty-two patients from 11 clinics underwent eFCU with one unsuccessful exam making the failure rate 2.3% (95% CI 0.0006–0.1256). Addition of eFCU led to a significant increase in volume of patients able to be seen 19 versus 15.5 (p < 0.01). A majority of parents/patients reported a positive experience with eFCU.Conclusion:Expert focused cardiac ultrasound with spectral Doppler can be used successfully for follow-up in patients with select CHD and the addition of eFCU permits increased patient capacity in outreach clinics and has the potential to improve the patient experience.

To test the hypothesis that right ventricular (RV) function has age-specific patterns of development, we tracked the evolution of RV strain mechanics by 2D-speckle tracking echocardiography (2DSTE) in healthy subjects from mid-gestation through one year of age.We conducted a prospective longitudinal echocardiography study in 50 healthy subjects at five time periods across gestation (16-20 weeks, 21-25 weeks, 26-30 weeks, 31-35 weeks, and 36-40 weeks) and four time periods following delivery (1 week, 1 month, 6 months, and 1 year of age). We characterized RV function by measuring RV global and free wall longitudinal strain and systolic strain rate, and segmental longitudinal strain at the apical-, mid-, and basal- ventricular levels of the free wall. Possible associations of gestational age, postnatal age, estimated fetal weight, body surface area, gender, and heart rate on strain were investigated.The magnitudes of RV global and free wall longitudinal strain and global strain rate were decreased throughout gestation (p 0.05 for all). Following birth, the magnitudes of all measures increased from one week through one year (p 0.001 for all). RV segmental longitudinal strain maintained a base-to-apex gradient (highest-to-lowest) from mid-gestation through one year (p 0.001). There was no significant difference in strain patterns based on gender or hear rate.The maturational patterns of RV strain are gestational- and postnatal age- specific. With accepted physiological maturation patterns in healthy subjects, these myocardial deformation parameters can provide a valid basis that allows comparison between health and disease.

Highlights • Patterns of energy loss in the LV differ among subjects with HCM, DCM, and normal controls. • Our study demonstrated significant elevation in diastolic WSS and indexed systolic EL in HCM. • Few differences were observed in energetic parameters in DCM. • Ventricular energetics may represent novel indices of ventricular diastolic dysfunction., Background Vector flow mapping is an emerging echocardiographic method allowing for investigation of intracardiac blood flow mechanics, wall shear stress (WSS), and energy loss (EL). We hypothesized that alterations in EL and WSS will differ among subjects with hypertrophic (HCM), dilated (DCM) cardiomyopathy, and normal controls. Methods Echocardiograms were prospectively performed with the ProSound F75CV (Hitachi HealthCare., Tokyo, Japan) on all subjects. 2D color Doppler cine loop images were obtained from apical 5 and the apical long axis views and stored digitally. Measurements were averaged over three cardiac cycles using VFM software to derive flow patterns, WSS, and EL. Standard left ventricular (LV) systolic and diastolic functional parameters were also obtained. Results A total of 85 subjects, 22 with HCM (age 18 ± 9 yrs.), 18 DCM (age 18 ± 9 yrs.), and 45 age and gender matched controls were included in the study. Diastolic wall shear stress was found significantly different in HCM (0.004 ± 0.185 N/m2) compared with DCM (0.397 ± 0.301 N/m2, P

Patients with the Turner syndrome (TS) often have longer QT intervals compared with age-matched peers although the significance of this remains unknown. We sought to determine the degree, frequency and impact of QTc prolongation in patients with TS. A chart review of all patients with an electrocardiogram (ECG) and genetically proven TS was performed. Medications at the time of the ECG were reviewed and QTc calculated. Medications were classified according to QTc risk using www.crediblemeds.com. ECG parameters were compared with an age, gender, and cardiac lesion-matched control group. Over the 10-year period of review, 112 TS patients with a mean age of 34 ± 25 years underwent 226 ECGs. At least 1 QTc prolonging medication was prescribed in 81 (74%) patients. Longer QTc interval correlated with absence of y chromosomal material (p = 0.01), older age (p0.0001), increased number of QTc prolonging and nonprolonging medications (p0.0001 each). During the 7.0 ± 5.1 years of follow-up, no patient had ventricular arrhythmia or unexplained sudden death. QTc was significantly shorter in matched controls using either Bazett or Hodges formula (424 ± 16 ms vs 448 ± 28 ms, p0.0001; and 414.8 ± 16 ms vs 424.2 ± 20 ms; p = 0.0002, respectively). However, there was no difference in the frequency of QTc prolongation460 msec (2.8% vs 2.6%, p = 0.9). In conclusion, despite frequent use of QT-prolonging medications, ventricular arrhythmias are rare in TS.

BACKGROUND Changes in 2D echocardiography (2DE) speckle tracking imaging (STI) derived left ventricular (LV) strain (S) and strain rate (SR) precedes diminution of LV ejection fraction (LVEF) in adult valvular aortic stenosis (AS). We prospectively examined whether 2DE-STI derived multidirectional LV S and SR correlate with AS severity in children using LV mass index (MI) as the principal outcome variable. METHODS 52 children (10.4 ± 7.3 years) with isolated congenital AS were included; 13 mild (2.5 m/s 4.0 m/s). 2DE including Doppler and STI longitudinal strain (LS), strain rate (LSR), circumferential strain (CS), and strain rate (CSR) were measured. Univariate and multivariable linear regressions identified correlations between LVMI and strain indices. RESULTS Three clinical and 2DE variables, and four strain indices were independently associated with LVMI. LVMI correlated positively with systolic blood pressure and aortic regurgitation, and negatively with LVEF. LVMI correlated positively with LSR (four-chamber) and CSR (basal), and negatively with segmental CS in the inferior (basal) and anteroseptal (distal) segments. LVMI showed significant inverse association with LS (P = .05), LSR (P

The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.

This study evaluated hepatic stiffness by shear wave elastography to investigate subclinical hepatic changes in a cohort of patients with congenital biventricular heart disease (BHD).The BHD patients and age-matched healthy controls were prospectively recruited for hepatic ultrasonography and shear wave elastography. Real-time B-mode imaging with Doppler was performed for celiac axis, superior mesenteric artery, and main portal vein, and hepatic shear wave elastography was assessed. Vascular Doppler indices included peak velocities; velocity time integral, resistive, pulsatility, and acceleration indices; and portal vein volumetric flow. One-way analysis of variance was used for comparisons between controls, BHD, and a cohort of Glenn and Fontan patients.In all, 66 subjects were included. Thirty-six subjects were in the BHD group (male, 25; female, 11; mean age 27.4 ± 4.6 years; mean weight 76.8 ± 18.5 kg), and 30 were healthy controls (male, 11; female, 23, mean age 27.4 ± 3.8 years; mean weight 70 ± 17.2 kg). Shear wave elastography was increased in BHD (8.11 ± 2.07 kPa) compared with controls (5.44 ± 1.18 kPa; P.001). Hepatic stiffness in BHD was significantly different from that in the Fontan cohort but not in the infant Glenn cohort.Increased hepatic stiffness was observed in young adults with repaired BHD. Although cause is not established, possibilities include hepatic congestion early in life or elevated central venous pressures due to right heart burden. Further research is required to determine whether these patients will ultimately have clinically relevant liver disease.

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex ‘double switch’ repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.

Background Congenital heart disease (CHD) is the most common type of major congenital malformations. CHD outcomes have improved in the past 20 years, and research efforts in the field have increased exponentially. The purpose of this investigation is to identify the most influential recent publications related to CHD, and reveal their shared features using bibliometric analysis. Methods Journal articles related to CHD published between 2000 and 2020 were retrieved from the Scopus database. In order of citation frequency, the 100 most-cited clinical original articles overall, the 20 most-cited clinical original articles of the years 2018–2020, and the top 10 most-cited review articles were compiled. The lists were analyzed to identify characteristics of highly cited publications. VOS Viewer was used to construct and visualize network maps based on co-authorship and co-occurring keywords. Results The total number of citations varied from 244 to 1741, and the number of citations per year varied from 13.2 to 121.9. Articles were most commonly published in Circulation, Journal of the American College of Cardiology, or Journal of Thoracic and Cardiovascular Surgery. Most originated from the USA, and had a sample size of 200 patients or less. A total of 46 authors had at least 3 publications among the list and were organized in 6 collaboration clusters. Tetralogy of Fallot, atrial septal defect, the perinatal period, Fontan, and cardiac surgery were identified as major areas of research. Conclusions This bibliographic analysis mapped two decades of research in CHD, highlighting important articles and defining major areas of research.

Inertial cavitation inducing ultrasound-mediated microbubble treatments can produce resolution of vasospasm and restoration of distal arterial flow after peripheral artery injury. Resolution of catheter-induced vasospasm is likely to be nitric oxide- mediated because improvements in stenosis diameter and downstream blood flow were blunted following pretreatment with L-NAME. The potential for clinical applicability of this therapy is significant because: 1) microbubbles can be delivered systemically into the site of injury enabling relatively high local concentration; 2) targeted transcutaneous ultrasound delivery is achievable due to the proximity of vessels; and 3) microbubbles and diagnostic ultrasound system used are commercially available.

Background We hypothesized that right atrial (RA) performance is abnormal in repaired tetralogy of Fallot (TOF). Methods TOF patients were prospectively enrolled for cardiovascular magnetic resonance (CMR), echocardiography and exercise stress following a standardized 14-center protocol. Peak RA longitudinal strain (RALS) and right ventricular longitudinal strain (RVLS) were measured using CMR feature tracking (FT) and correlated to RA and RV end diastolic volumes (EDVi) and ejection fraction (EF). Results The cohort had 311 subjects: 171 TOF (94 male, age 18.2±8years) and 140 healthy controls (69 male, 16.4±11years). RAEDVi, RALS, RVEDVi, RVLS, RAEF, and RVEF in TOF were 60.8±17.1ml/m 2 , 13.6±5.7%, 120.3±30.3ml/m 2 , 12.3±4.2%, 32.5±9.9% and 51.2±8.4% and differed from respective indices in controls: 51.7±15.7ml/m 2 , 27±10.1%, 74±19.0ml/m 2 , 18.5±5.3%, 54±8% and 62.5±5.5% (p Conclusion RALS, RAEDVi and RAEF are abnormal in TOF. Reduced RALS indicates decreased RA reservoir function. Because they correlate with other functional RV indices, these abnormalities may represent RA diastolic burden from chronic RV dysfunction in TOF. The young cohort age might explain the absence of RALS correlation to tricuspid regurgitation and peak oxygen uptake.

Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service.Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters.Single freestanding pediatric hospital located in the central United States.Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter.Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data.Twelve patients had a total of 19 transhepatic venous lines. Transhepatic venous lines had a significantly longer duration of service than central venous lines (p = 0.001). No difference between the two groups was found in the number of documented thrombi, thrombolytic burden, or catheter sites requiring wound care consultation. A higher frequency of infection in transhepatic venous lines versus central venous lines was found, isolated to four transhepatic venous lines that had a total of nine infections. All but one was successfully managed without catheter removal. The difference in the proportion of infections to catheters in transhepatic venous lines versus central venous lines was significant (p = 0.0001), but no difference in the rate of infection-related catheter removal was found.Without compromising future central venous access sites, transhepatic venous lines had superior duration of service without increased thrombosis, thrombolytic use, or insertion site complications relative to central venous lines. Transhepatic venous catheters had a higher infection rate, and further investigation into the etiology is warranted.

Left ventricular two-dimensional global longitudinal strain (LS) is superior to ejection fraction (EF) as predictor of outcome. We provide reference data for atrial and ventricular global LS during childhood and adolescence by CMR feature tracking (FT). We prospectively enrolled 115 healthy subjects (56 male, mean age 12.4 ± 4.1 years) at a single institution. CMR consisted of standard two-dimensional steady-state free-precession acquisitions. CMR-FT was performed on ventricular horizontal long-axis images for derivation of right and left atrial (RA, LA) and right and left ventricular (RV, LV) peak global LS. End-diastolic volumes (EDVs) and EF were measured. Correlations were explored for LS with age, EDV and EF of each chamber. Mean±SD of LS (%) for RA, RV, LA and LV were 26.56±10.2, -17.96±5.4, 26.45±10.6 and -17.47±5, respectively. There was a positive correlation of LS in LA, LV, RA and RV with corresponding EF (all P

OBJECTIVE Experimental evidence suggests that changes in the fetal myocardium result from intrauterine effects of maternal diabetes mellitus and obesity. The aim of this study was to assess fetal cardiac function using two-dimensional speckle-tracking echocardiography to determine the effects of maternal diabetes and obesity on the fetal myocardium. METHODS Comparative cross-sectional evaluation of myocardial function in fetuses of mothers with diabetes mellitus (FDM) or obesity (FO) and normal gestational age-matched control fetuses (FC) was performed using two-dimensional speckle-tracking echocardiography at two centers. RESULTS In total, 178 fetuses (82 FDM, 26 FO and 70 FC) met the enrolment criteria. Mean gestational age at assessment was similar among groups: 25.3 ± 5.1 weeks for FDM, 25.0 ± 4.6 weeks for FO and 25.1 ± 4.9 weeks for FC. Mean maternal body mass index was significantly higher in FDM and FO groups compared with the FC group. Statistically significant differences in fetal cardiac function were detected between FDM and FC for global longitudinal strain (mean ± SD, -21.4 ± 6.5% vs -27.0 ± 5.2%; P

Echocardiographic assessment of right atrial (RA) volume, inferior vena cava (IVC) diameter, and hepatic vein flow velocity independently correlate with the RA pressure by direct catheter measurement in adults. We prospectively collected invasive RA pressure measurements and echocardiographic data in infants and young children with the goal of developing a predictive model to noninvasively determine normal RA pressure. All subjects had a central venous catheter through which RA pressure could be transduced. Specific inclusion criteria consisted of (1) biventricular heart, (2) absence of inotropes, (3) sinus rhythm, and (4) at least 24 hours from surgery. Two-dimensional echocardiography (2DE)-Doppler and 3DE-Doppler were used to measure RA volume, systemic venous diameters, and flow velocity. Regression equations of RA pressure with RA volume, systemic venous size, and flow velocity were explored. Of 46 studies, 43 (93%) had echocardiograms adequate for analysis. RA pressure did not correlate with body surface area or age (p = 0.69, p = 0.87). The mean indexed RA volume by 3DE-Doppler was significantly higher than by 2DE (p 2 ). RA pressure did not show correlation with systemic venous diameters or systolic and diastolic flow velocities in the SVC and hepatic veins. In conclusion, regression incorporating 2DE-derived RA volume and IVC systolic flow velocity provided the best noninvasive estimate of normal RA pressure in infants and children. The model derived requires validation in an independent sample.

Background and objectives Our purpose was to investigate cardiovascular abnormalities in children with osteogenesis imperfecta (OI). Methods Two hundred children (100 OI, 100 matched volunteers) were prospectively studied. Aortic and left ventricular (LV) measurements were performed using transthoracic echocardiography. Patients were typed according to modified phenotypical Sillence classification as published in the Nosology and Classification of Genetic Skeletal disorders: 2015 Revision. Results Patients (age 9.6±4.1 years, body surface area 1.08±0.47 m2) consisted of OI types: 1 (n=44), 3/4 (n=54), 4 (n=1) and 15 (n=1). The 95% CIs for Z­score of aortic annulus, sinus, sinotubular junction and ascending aorta for OI were 0.43 to 0.73, 0.56 to 0.94, 0.28 to 0.70 and 0.78 to 1.24, respectively. In type 1, sinus, sinotubular junction and ascending aorta diameters were 2.29 cm, 1.81 cm and 2.05 cm, respectively, which did not differ compared with controls. The LV dimensions were larger in type 1. In type 3/4, aortic dimensions were larger than controls at all levels: annulus (1.61 vs 1.50 cm, p Conclusions Cardiovascular effects are identifiable in childhood even in mild forms of OI. Aortic dilation was the predominant finding, while valvular abnormalities were infrequent. Patients with more severe skeletal pathology (types 3/4) have more significant findings. Aortic and LV dilation in type 1 vs type 3/4 appears to differ based on the biochemical mechanism of disease.

Purpose We used two-dimensional echocardiographic speckle-tracking to investigate whether left and right atrial (LA and RA) phasic function in pediatric heart transplantation (HT) patients is altered and explored the relationship to HT-related clinical variables. Methods Eighty-six subjects (36 HT and 50 normal children) were prospectively enrolled in two centers. Clinical data included age at HT, bypass time, ischemia time, donor age, and incidence of rejection. Atrial deformation indices including strain and strain rates (SRs) were measured using two-dimensional echocardiographic speckle-tracking. Components of phasic atrial function—reservoir (er, SRr), conduit (ecd, SRcd), and booster (ect, SRct) were calculated. Comparisons with controls were made using t test or Kruskal-Wallis test, and correlations to clinical variables were explored. Results The mean age and body surface area of HT subjects were 10.2 ± 6.2 years and 1.2 ± 0.6 m2, respectively. The mean heart rates were higher in HT (96 ± 18 vs 88 ± 21 in controls). There were reductions in RA and LA reservoir (er, SRr), conduit (ecd, SRcd), and booster (ect, SRct) function in HT compared with controls. There was no relationship of LA and RA deformation indices with mean age at HT, bypass time, or ischemia time. The LA ecd correlated weakly with donor age (r = –0.49, P = .04) and RA SRr, and SRcd showed association with duration of HT (P Conclusions Atrial strain determination is feasible in pediatric HT recipients and demonstrates disruption of reservoir, conduit, and booster function of both atria in this population; we speculate this may be a consequence of ventricular diastolic dysfunction.

Quantification of pulmonary regurgitation (PR), pulmonary flow distribution, and ventricular function is important for clinical surveillance in repaired Tetralogy of Fallot (TOF). Cardiovascular magnetic resonance (CMR) is the established reference, but cost, test duration, and patient discomfort are potential limitations to its serial use. We investigated whether an Abbreviated CMR protocol would alter clinical decisions in TOF from those that would have been made using a full protocol. Patients > 7 years with repaired TOF were identified. CMR was performed according to standard complete imaging protocol. CMRs were prepared in two ways, Full and Abbreviated and submitted for review by two imaging specialists. In conjunction with clinical information and case-specific quantitative CMR data (PR fraction, ventricular volumes, ejection fraction, branch pulmonary artery flow), Full and Abbreviated image sets were anonymized and uploaded for review. For the first half, Imager 1 received Abbreviated, and Imager 2 Full and for the remaining, Imager 1 received Full and Imager 2 received Abbreviated. Blinded to the other’s choices, Imagers provided clinical decisions. Inter-rater agreement for each decision was measured. In all, 124 studies from 80 patients (mean 17.8 years) were analyzed. For ‘intervention versus no-intervention’ decision, the inter-rater agreement was strong [κ 0.75, p

To the Editor We thank the correspondents for their insightful comments1 regarding the benefits of pharmacotherapy in selected patients with systemic right ventricles. The underlying principles of pharmacologic management of the systemic right ventricle are necessarily based on less than robust clinical experience, but are probably similar to those guiding management of congestive left ventricular failure. Therefore, we also use ACE inhibitors for …

Patients with atrioventricular nodal reentry tachycardia (AVNRT) often are managed successfully by ablation of the slow pathway with success rates reported as high as 99%. Low voltage bridges (LVBs) have been demonstrated to be helpful in guiding AVNRT ablation. Patients may present to the electrophysiology lab without evidence of inducible arrhythmia. In these scenarios, the demonstration of LVBs may be diagnostic and guide catheter ablation treatment. The purpose of our study was to prospectively investigate the specificity of LVBs as a diagnostic marker of AVNRT. Patients aged

The Fontan operation for single ventricle palliation consists of the creation of a complete cavopulmonary connection, usually by incorporating inferior vena caval flow into a pulmonary arterial circulation already receiving flow from the superior vena cava. In single ventricle palliated in this way, the anatomy is complex, and the pathophysiological complications are frequent; so, cardiac imaging is a key aspect of clinical surveillance. Common problems that echocardiography and MRI may disclose and characterise in the Fontan palliation of single ventricle include obstruction of systemic venous and pulmonary arterial flow, atrioventricular and semilunar valve dysfunction, unintended collateral flow patterns, ventricular dysfunction, aortic arch obstruction, interatrial obstruction, fenestration flow and patch leaks. Despite the broad scope of these modalities for detection of such problems, often no single imaging method is comprehensive in any given patient. Therefore, physicians must recognise the limitations of each modality, and circumvent these by application of suitable alternatives. New imaging tools are becoming available, which may ultimately prove to be of value in the Fontan circulation. Proper application of diverse new technologies such as four dimensional flow, computational fluid dynamics and three-dimensional printing will require critical evaluation in the single ventricle population.

Background Predischarge (pre-d/c) transthoracic echocardiography (TTE) is routine after surgery for congenital heart disease, but how it affects clinical care is unknown. The aim of this study was to test the hypothesis that pre-d/c TTE frequently reveals findings associated with short-term clinical course through a systematic review of findings on pre-d/c TTE and clinical events that followed. Methods Clinical outcomes of mortality, hospitalization, catheterization, and surgery at 1 year were examined for pediatric patients undergoing pre-d/c TTE between June 2010 and June 2012. Using logistic regression, a multivariate model was generated associating clinical, pre-d/c transthoracic echocardiographic, and demographic variables with unplanned postdischarge cardiac events (UCEs) within 1 year. Results Of 462 patients who underwent pre-d/c TTE, there were 265 male patients (57%) and 197 female patients (43%); the median age was 0.8 years (range, 0–33 years). Two hundred thirty–seven patients (51%) had findings (valve regurgitation, hemodynamic obstruction, ventricular dysfunction, unintended shunt, or pericardial effusion) on pre-d/c TTE, 57 of which were of more than mild severity. Agreement between pre-d/c TTE and postoperative transesophageal echocardiographic findings was only fair to moderate (κ = 0.27–0.43). Sixty-four patients (14%) had UCEs. Univariate analysis revealed that UCE were more frequent in patients with diagnoses and surgical procedures of high complexity. After accounting for these confounding nonechocardiographic variables, pre-d/c transthoracic echocardiographic findings, specifically valve regurgitation of more than mild severity, and ventricular dysfunction and obstructions of any severity were independently associated with UCEs (odds ratios, 1.90, 1.99, and 1.85, respectively). Conclusions Findings on pre-d/c TTE are frequent, commonly discordant with postoperative transesophageal echocardiographic results, and associated with adverse clinical events after surgery for congenital heart disease. These data would strongly support the practice of pre-d/c TTE after surgery for congenital heart disease.